Angela Miniaci

Long-term Clinical Significance of Thyroid Autoimmunity in Children with Celiac Disease

OBJECTIVE To evaluate the long-term outcome of thyroid function and autoimmunity in a large series of children with celiac disease. STUDY DESIGN This longitudinal, retrospective study (duration of follow-up, 8.9 +/- 4.0 years) was conducted at the Pediatric Department, University of Bologna, Italy. One hundred thirty-five consecutive patients diagnosed between June 1990 and December 2004 and followed on a gluten-free diet were examined. Inclusion criteria were good dietary compliance and duration of follow-up for at least 3 years. RESULTS Of 101 patients who never showed positive antithyroid titers during the follow-up, 86 remained euthyroid; 15 showed high thyroid-stimulating hormone values at diagnosis that normalized in 11 cases after 12 to 18 months of gluten withdrawal. Of 31 patients with persistently positive antibody titers, 23 (74%) remained consistently euthyroid during the follow-up and 8 (26%) had a subclinical hypothyroidism. The prevalence of cases with positive antibodies was similar in children with growth retardation or gastroenterological symptoms at diagnosis and different durations of gluten exposure. CONCLUSIONS The presence of antithyroid antibodies in children with celiac disease has a low predictive value for the development of thyroid hypofunction during the indicated surveillance period. Longer follow-up is needed.

Renal involvement in hypocomplementaemic urticarial vasculitis syndrome: a report of three paediatric cases

OBJECTIVE To describe the diagnosis and management of renal disease in three paediatric cases of hypocomplementaemic urticarial vasculitis syndrome (HUVS). METHODS Three children who were diagnosed with HUVS and developed abnormalities of renal function during the disease course are described. RESULTS Urinary findings were heterogeneous: all the patients developed persistent microhaematuria, which was isolated in patient 1, associated with mild proteinuria in patient 2 and with nephrotic syndrome in patient 3. Renal biopsies were performed in all the patients: patients 1 and 2, who had normal levels of serum autoantibodies, shared a full-house IF (C3, C1q and Ig deposits), compatible with an SLE-like disease; patient 3 showed negative staining for IgG and IgM, but developed positive anti-dsDNA without fulfilling criteria for the diagnosis of SLE. CONCLUSION Renal involvement in HUVS is probably more frequent and more severe than in adults and may appear later. Isolated microhaematuria can be the only sign of subclinical renal involvement: its role should not be underestimated and a renal biopsy should be considered. Previous observations of rapidly progressive nephritis and consequent end-stage renal disease in children suggest the need for prompt diagnosis and treatment of renal involvement.

Evolution of hypogammaglobulinemia in premature and full-term infants.

There are few data in the literature reporting the evolution of hypogammaglobulinemia in premature and full-term infants during the first years of life. The aim of this study was to assess the clinical and immunological evolution of premature and full-term infants with hypogammaglobulinemia. We included 24 children (11 premature and 13 full-term infants), aged 0–36 months, with hypogammaglobulinemia. Fifteen (62.5%) children had an isolated reduction in IgG, 7 (29.2%) had a decrease in both IgG and IgA and 2 (8.3%) a reduction in IgG and IgM. Normalization of IgG serum levels occurred in the premature infants at a mean age of 7.2 months. Full-term infants were divided into 3 groups based on age at normalization of IgG serum level: A) hypogammaglobulinemia with normalization within 12 months of life; B) with normalization within 36 months of life; C) normalization after 36 months. All the premature infants with hypogammaglobulinemia recovered, even though in the lower limits for age in the first years, while transient hypogammaglobulinemia observed in full-term infants has a different age of recovery.

Correction to: The Italian version of the Juvenile Arthritis Multidimensional Assessment Report (JAMAR)

The family name of author Francesco La Torre was incorrect in the published article. The correct family name should read as La Torre F.

The Italian version of the juvenile arthritis multidimensional assessment report (JAMAR)

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Italian language.The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents.The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the 3 Likert assumptions, floor/ceiling effects, internal consistency, Cronbach’s alpha, interscale correlations, test–retest reliability, and construct validity (convergent and discriminant validity).A total of 1296 JIA patients (7.2% systemic, 59.5% oligoarticular, 21.4% RF negative polyarthritis, 11.9% other categories) and 100 healthy children, were enrolled in 18 centres. The JAMAR components discriminated well healthy subjects from JIA patients except for the Health Related Quality of Life (HRQoL) Psychosocial Health (PsH) subscales. All JAMAR components revealed good psychometric performances.In conclusion, the Italian version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research.

Cerebral Ischemic Involvement in Vogt-Koyanagi-Harada Disease

BACKGROUND Vogt-Koyanagi-Harada (VKH) disease is an autoimmune disorder characterized by ocular, auditory and neurological manifestations (headache, meningismus and/or aspeptic meningoencephalitis). PATIENT We describe a 12-year-old African boy with bilateral uveitis who presented with acute unilateral hearing loss and neurological symptoms such as left-sided dyskinesias, unsteady gait and throbbing headache. Brain magnetic resonance imaging showed ischemic lesions of the right basal ganglia in the territory of lenticulostriate and thalamic arteries. He improved after treatment with intravenous and oral steroids. CONCLUSION Cerebral ischemic episodes should be included in the possible neurological manifestations of VKH.

PReS-FINAL-2022: Juvenile idiopathic arthritis after allogeneic bone marrow transplantation: a case report

The allogeneic bone marrow transplantation is a therapeutic weapon for treating severe and drug-resistant autoimmune diseases determining the resolution and improvement in the quality of life. On the other hand autoimmune disorders can develop after a hematopoietic stem cell transplantation (HSCT).